Background The Scale for the Assessment and Rating of Ataxia (SARA) was shown to be a reliable and valid measurement for patients with spinocerebellar ataxia (SCA). The Brazilian version and the Japanese version o...Background The Scale for the Assessment and Rating of Ataxia (SARA) was shown to be a reliable and valid measurement for patients with spinocerebellar ataxia (SCA). The Brazilian version and the Japanese version of SARA were favorable for good reliability and validity. This study aimed to translate SARA into Chinese and test its reliability and validity in measurement of cerebellar ataxia. Methods SARA was translated into Chinese. A total 39 patients with degeneration cerebellar ataxia were evaluated independently by two neurologists with the Chinese version of SARA. Then the patients were evaluated by one of above neurologists with International Cooperative Ataxia Rating Scale (ICARS). The statistical analyses were performed using SPSS 17.0 for Windows. Results The Cronbach's alpha coefficient of the Chinese version of SARA was 0.78, which represents a good internal consistence. The correlation coefficient of the Chinese version of SARA scores between the two evaluators was 0.86, illustrating that the inter-rater reliability of Chinese version of SARA was good. The correlation coefficient between the Chinese version of SARA and ICARS was 0.91, illustrating that the criterion validity of Chinese version of SARA was not bad. Conclusions The Chinese version of SARA is reliable and effective for the assessment of degeneration cerebellar ataxia. Compared with ICARS, the evaluation of Chinese version of SARA is more objective, the assessment time is shortened, and the maneuverability is better.展开更多
BACKGROUND Psychological factors such as anxiety and depression will not only aggravate the symptoms of chronic obstructive pulmonary disease(COPD)patients and reduce the quality of life of patients,but also affect th...BACKGROUND Psychological factors such as anxiety and depression will not only aggravate the symptoms of chronic obstructive pulmonary disease(COPD)patients and reduce the quality of life of patients,but also affect the treatment effect and long-term prognosis.Therefore,it is of great significance to explore the clinical application of senile comprehensive assessment in the treatment of COPD and its influence on psychological factors such as anxiety and depression.AIM To explore the clinical application of comprehensive geriatric assessment in COPD care and its impact on anxiety and depression in elderly patents.METHODS In this retrospective study,60 patients with COPD who were hospitalized in our hospital from 2019 to 2020 were randomly divided into two groups with 30 patients in each group.The control group was given routine nursing,and the observation group was given comprehensive assessment.Clinical symptoms,quality of life[COPD assessment test(CAT)score],anxiety and depression Hamilton Anxiety Rating Scale(HAMA)and Hamilton Depression Rating Scale(HAMD)were compared between the two groups.RESULTS CAT scores in the observation group decreased from an average of 24.5 points at admission to an average of 18.3 points at discharge,and in the control group from an average of 24.7 points at admission to an average of 18.3 points at discharge.The average score was 22.1(P<0.05).In the observation group,HAMA scores decreased from 14.2 points at admission to 8.6 points at discharge,and HAMD scores decreased from 13.8 points at admission to 7.4 points at discharge.The mean HAMD scores in the control group decreased from an average of 14.5 at admission to an average of 12.3 at discharge,and from an average of 14.1 at admission to an average of 11.8 at discharge.CONCLUSION The application of comprehensive geriatric assessment in COPD care has a significant effect on improving patients'clinical symptoms and quality of life,and can effectively reduce patients'anxiety and depression.展开更多
Assessment of locomotion recovery in preclinical studies of experimental spinal cord injury remains challenging. We studied the CatWalk XT■gait analysis for evaluating hindlimb functional recovery in a widely used an...Assessment of locomotion recovery in preclinical studies of experimental spinal cord injury remains challenging. We studied the CatWalk XT■gait analysis for evaluating hindlimb functional recovery in a widely used and clinically relevant thoracic contusion/compression spinal cord injury model in rats. Rats were randomly assigned to either a T9 spinal cord injury or sham laminectomy. Locomotion recovery was assessed using the Basso, Beattie, and Bresnahan open field rating scale and the CatWalk XT■gait analysis. To determine the potential bias from weight changes, corrected hindlimb(H) values(divided by the unaffected forelimb(F) values) were calculated. Six weeks after injury, cyst formation, astrogliosis, and the deposition of chondroitin sulfate glycosaminoglycans were assessed by immunohistochemistry staining. Compared with the baseline, a significant spontaneous recovery could be observed in the CatWalk XT■parameters max intensity, mean intensity, max intensity at%, and max contact mean intensity from 4 weeks after injury onwards. Of note, corrected values(H/F) of CatWalk XT■parameters showed a significantly less vulnerability to the weight changes than absolute values, specifically in static parameters. The corrected CatWalk XT■parameters were positively correlated with the Basso, Beattie, and Bresnahan rating scale scores, cyst formation, the immunointensity of astrogliosis and chondroitin sulfate glycosaminoglycan deposition. The CatWalk XT■gait analysis and especially its static parameters, therefore, seem to be highly useful in assessing spontaneous recovery of hindlimb function after severe thoracic spinal cord injury. Because many CatWalk XT■parameters of the hindlimbs seem to be affected by body weight changes, using their corrected values might be a valuable option to improve this dependency.展开更多
Nowadays, assessing speaking has received a great degree of attention not only because it is difficult to develop and administer, but also it plays a significant role in eliciting the purpose of foreign language learn...Nowadays, assessing speaking has received a great degree of attention not only because it is difficult to develop and administer, but also it plays a significant role in eliciting the purpose of foreign language learning. Studies on assessing speaking explored different proposals influencing the test of speaking, such as the nature of speaking, basic types of speaking, mode of test and rating scales. This study compares different proposals for assessing speaking in foreign language learning. Deep analyses according to Beijing Oral English Certificate(BOEC) testing will be presented with the improvement suggestions.展开更多
AIM: To investigate child and adolescent psychiatrists'(CAPs) attention deficit hyperactivity disorder(ADHD) and oppositional defiant disorder(ODD) diagnoses and treatments in real-world clinical practice. METHODS...AIM: To investigate child and adolescent psychiatrists'(CAPs) attention deficit hyperactivity disorder(ADHD) and oppositional defiant disorder(ODD) diagnoses and treatments in real-world clinical practice. METHODS: The medical records of 69 ADHD children(mean age = 9.5 years), newly referred to the ADHD clinic, were reviewed for their scores of parent- and teacher-reported Vanderbilt ADHD Diagnostic Rating Scales(VADRSs), CAPs' diagnoses of ADHD and ODD, and CAPs' treatment recommendations. Among 63 ADHD subjects who completed both parent and teacher VADRSs, we examined the agreement of the parent and teacher VADRSs. We also examined the concurrent validity of CAPs' ODD diagnoses against the results from the VADRSs. In addition, we compared CAPs' treatment recommendations against established ADHD and ODD guidelines.RESULTS: Among 63 ADHD subjects, the majority of the subjects(92%) met full ADHD diagnostic criteria at least in one setting(parent or teacher) on the VADRSs. Nearly half of the patients met full ADHD diagnostic criteria in two settings(parent and teacher). Relatively low agreement between the parent and teacher VADRSs were found(95%CI:-0.33 to 0.14). For 29 children who scored positive for ODD on the rating scales, CAPs confirmed the ODD diagnosis in only 12 of these casepositives, which is considered as a fair agreement between CAPs and VADRSs(95%CI: 0.10-0.53). For 27 children with no ODD diagnosis made by either CAP or VADRS, more than half of them were recommended for medication only. In contrast, where CAPs made the diagnosis of ODD, or where the parent or teacher VADRS was positive for ODD, almost all of the patients received recommendations for medication and behavior therapy.CONCLUSION: CAPs' ADHD diagnoses have strong concurrent validity against valid rating scales, but ADHD's most common comorbid condition- ODD- may be underrecognized.展开更多
Few experimental studies have evaluated the efficacy of continuing educational programs aimed at the improvement of nurses’ pain-management skills. This study assessed whether a standardized educational program aimed...Few experimental studies have evaluated the efficacy of continuing educational programs aimed at the improvement of nurses’ pain-management skills. This study assessed whether a standardized educational program aimed at nurses could increase the use of the Numeric Rating Scale-11 in both documenting and reducing postoperative pain-intensity levels in hospitalized surgical patients. The study had a quasi-experimental pre- and post-intervention design. Data were collected from records of surgical patients prior to and after the standardized educational program was completed. There were no significant differences between pre- and post-intervention groups in terms of either pain-documentation frequency or pain-intensity level. The study showed no increase in the frequency of postoperative pain documentation and no reduction of surgical patients’ postoperative pain-intensity level. This finding indicates that the standardized educational program on postoperative pain management was insufficient to bring about changes in clinical practice.展开更多
Ataxias are rare diseases and the etiologic heterogeneity make individual entities even rarer.There are still substantial numbers of patients who are still poorly understood.Available assessment techniques still point...Ataxias are rare diseases and the etiologic heterogeneity make individual entities even rarer.There are still substantial numbers of patients who are still poorly understood.Available assessment techniques still point to large numbers of patients needed for clinical trials and the need for cooperative efforts,better assessment tools and novel trial designs.Better understanding of neural circuitry abnormalities may lead to more effective symptomatic therapy.Opportunities exist for targeting at risk individuals for effective therapies but how this can be done is not clear.Preventive strategies may become feasible in many ataxias.展开更多
Objective:To study the therapeutic effect of Shangjinbitong Powder(伤筋痹痛散)on improving acupoint plaster,in order to provide some scientific basis for clinical use.Methods:This project was carried out from June 202...Objective:To study the therapeutic effect of Shangjinbitong Powder(伤筋痹痛散)on improving acupoint plaster,in order to provide some scientific basis for clinical use.Methods:This project was carried out from June 2020 to January 2021,30 patients with low back pain in the outpatient department of orthopedics and traumatology in our hospital and 30 patients with low back pain in the rehabilitation department of Fuping County Hospital of Traditional Chinese Medicine were selected as the research objects.Patients with low back pain in our hospital were treated with Shangjinbitong Powder as the control group,and patients in rehabilitation department of Fuping County Hospital of Traditional Chinese Medicine as the research group were treated with acupoint plaster improved by Shangjinbitong Powder.After one week of treatment,the effective rates of the two groups were compared.Results:The effective rate of treatment in the study group was significantly higher than that in the control group(P<0.05).There were no adverse reactions such as infection,scald and bleeding in the study group and the control group during the treatment.Conclusion:Shangjinbitong Powder improved acupoint plaster has good curative effect,convenient use,certain safety and clinical popularization value.展开更多
Background: Spinocerebellar ataxia type 3 (SCA3) is the most common subtype of SCA worldwide, and runs a slowly progressive and unremitting disease course. There is currently no curable treatment available. Growing...Background: Spinocerebellar ataxia type 3 (SCA3) is the most common subtype of SCA worldwide, and runs a slowly progressive and unremitting disease course. There is currently no curable treatment available. Growing evidence has suggested that nerve growth factor (NGF) may have therapeutic effects in neurodegenerative diseases, and possibly also in SCA3. The objective of this study was to test the efficacy of NGF in SCA3 patients. Methods: We performed an open-label prospective study in genetically confirmed adult (〉18 years old) SCA3 patients. NGF was administered by intramuscular injection (18 μg once daily) fbr 28 days consecutively. All the patients were evaluated at baseline and 2 and 4 weeks after treatment using the Chinese version of the scale for assessment and rating of ataxia (SARA). Results: Twenty-one SCA3 patients ( 10 men and 11 women, mean age 39.14 ± 7.81 years, mean disease duration 4.14 ± 1.90 years, mean CAG repeats number 77,57 ± 2.27) were enrolled. After 28 days of NGF treatment, the mean total SARA score decreased significantly from a baseline of 8.48± 2.40 to 6.30 ± 1.87 (P 〈 0.001 ). Subsections SARA scores also showed significant improvements in stance (P = 0.003), speech (P = 0.023), finger chase (P = 0.015), fast alternating hand movements (P = 0.009), and heel-shin slide (P = 0.001). Conclusions: Our preliminary data suggest that NGF may be effective in treating patients with SCA3.展开更多
OBJECTIVE:To investigate the value of relative exchangeable copper(REC)in diagnosing Wilson's disease(WD)and to determine its significance in the differentiation of Traditional Chinese Medicine(TCM)syndrome.METHOD...OBJECTIVE:To investigate the value of relative exchangeable copper(REC)in diagnosing Wilson's disease(WD)and to determine its significance in the differentiation of Traditional Chinese Medicine(TCM)syndrome.METHODS:A total of 78 patients with WD were recruited on the same day of the medical visit,and among them,32 were suffering from non-WD(N-WD)and 37 were heterozygous ATP7B carriers(HC)enrolled as controls.Molecular genetic testing was performed for diagnosing WD and HC.Additionally,REC levels in different TCM syndromes were investigated.The correlation between REC and serum ceruloplasmin(Cp),serum copper oxidase(CO),and 24-h urinary copper was analyzed using the Global Assessment Scale and Unified Wilson's Disease Rating Scale and the significance of REC in WD diagnosis was investigated.Resting-state functional magnetic resonance imaging was used to assess the clinical symptoms of WD and analyze its severity in different TCM syndromes.RESULTS:REC determination helped in significantly distinguishing patients with N-WD or HC from those with WD with a cut-off of 21.15%.Furthermore,the comparative analysis of REC ratios among different TCM syndromes showed markedly high REC levels in the dampness-heat internal accumulation syndrome group.Additionally,the seed-based functional connectivity value from the putamen to the cerebellum was significantly correlated with TCM syndromes.CONCLUSION:REC level is a high-quality biomarker for diagnosing WD that effectively reflects disease severity and plays an essential role in deciding on treatment strategies and prognosis.Furthermore,REC levels are closely related to TCM syndromes in WD;thus,it is a potential objective quantitative indicator for distinguishing TCM syndromes in WD.展开更多
Objective:Vulvar lichen planus(VLP),especially erosive VLP(EVLP),seriously affects women’s physiological function and quality of life.A retrospective analysis of erosive vulvar lichen planus(EVLP)was performed to rai...Objective:Vulvar lichen planus(VLP),especially erosive VLP(EVLP),seriously affects women’s physiological function and quality of life.A retrospective analysis of erosive vulvar lichen planus(EVLP)was performed to raise awareness on this chronic,deforming,and painful vulvar disease.Methods:This observational retrospective study involved 40 patients with EVLP among 1,368 patients with vulvar disease from 2017 to 2020.The patients’vulvar clinical features and extravulvar mucosal involvement were investigated.The patients’pain and quality of life were evaluated with the numeric rating scale and the Dermatology Life Quality Index(DLQI),respectively.Clinical characteristics were reported using frequencies and proportions for categorical variables.Continuous variables are expressed as mean±standard deviation.The association between NRS score for pain and DLQI score was analyzed by Pearson’s correlation test.Results:EVLP accounted for 2.9%(40/1,368)of all cases of vulvar disease,and the median age at onset was 42 years.Erosion mostly occurred on the labia minora and introitus.Half(20/40)of the patients had extravulvar mucosal involvement.Seventeen(42.5%)patients had oral lichen planus,and seven(17.5%)patients were diagnosed with vulvovaginal-gingival syndrome.The numeric rating scale score showed that 47.5%(19/40)of patients experienced mild pain,whereas 12.5%(5/40)described the pain as severe.Thirty-one patients(77.5%,31/40)had dyspareunia,and nine patients had aggravated pain after sexual intercourse.The mean DLQI score was 9.1±6.0,with 37.5%(15/40)of patients indicating that EVLP had severely affected their lives.The numeric rating scale score for pain was significantly correlated with the DLQI score(P=0.007).Conclusion:EVLP can affect the quality of women’s lives and cause severe deformations and painful architectural changes.The description of the characteristics of EVLP in this report will benefit the diagnosis and early treatment of this disease.展开更多
文摘Background The Scale for the Assessment and Rating of Ataxia (SARA) was shown to be a reliable and valid measurement for patients with spinocerebellar ataxia (SCA). The Brazilian version and the Japanese version of SARA were favorable for good reliability and validity. This study aimed to translate SARA into Chinese and test its reliability and validity in measurement of cerebellar ataxia. Methods SARA was translated into Chinese. A total 39 patients with degeneration cerebellar ataxia were evaluated independently by two neurologists with the Chinese version of SARA. Then the patients were evaluated by one of above neurologists with International Cooperative Ataxia Rating Scale (ICARS). The statistical analyses were performed using SPSS 17.0 for Windows. Results The Cronbach's alpha coefficient of the Chinese version of SARA was 0.78, which represents a good internal consistence. The correlation coefficient of the Chinese version of SARA scores between the two evaluators was 0.86, illustrating that the inter-rater reliability of Chinese version of SARA was good. The correlation coefficient between the Chinese version of SARA and ICARS was 0.91, illustrating that the criterion validity of Chinese version of SARA was not bad. Conclusions The Chinese version of SARA is reliable and effective for the assessment of degeneration cerebellar ataxia. Compared with ICARS, the evaluation of Chinese version of SARA is more objective, the assessment time is shortened, and the maneuverability is better.
文摘BACKGROUND Psychological factors such as anxiety and depression will not only aggravate the symptoms of chronic obstructive pulmonary disease(COPD)patients and reduce the quality of life of patients,but also affect the treatment effect and long-term prognosis.Therefore,it is of great significance to explore the clinical application of senile comprehensive assessment in the treatment of COPD and its influence on psychological factors such as anxiety and depression.AIM To explore the clinical application of comprehensive geriatric assessment in COPD care and its impact on anxiety and depression in elderly patents.METHODS In this retrospective study,60 patients with COPD who were hospitalized in our hospital from 2019 to 2020 were randomly divided into two groups with 30 patients in each group.The control group was given routine nursing,and the observation group was given comprehensive assessment.Clinical symptoms,quality of life[COPD assessment test(CAT)score],anxiety and depression Hamilton Anxiety Rating Scale(HAMA)and Hamilton Depression Rating Scale(HAMD)were compared between the two groups.RESULTS CAT scores in the observation group decreased from an average of 24.5 points at admission to an average of 18.3 points at discharge,and in the control group from an average of 24.7 points at admission to an average of 18.3 points at discharge.The average score was 22.1(P<0.05).In the observation group,HAMA scores decreased from 14.2 points at admission to 8.6 points at discharge,and HAMD scores decreased from 13.8 points at admission to 7.4 points at discharge.The mean HAMD scores in the control group decreased from an average of 14.5 at admission to an average of 12.3 at discharge,and from an average of 14.1 at admission to an average of 11.8 at discharge.CONCLUSION The application of comprehensive geriatric assessment in COPD care has a significant effect on improving patients'clinical symptoms and quality of life,and can effectively reduce patients'anxiety and depression.
文摘Assessment of locomotion recovery in preclinical studies of experimental spinal cord injury remains challenging. We studied the CatWalk XT■gait analysis for evaluating hindlimb functional recovery in a widely used and clinically relevant thoracic contusion/compression spinal cord injury model in rats. Rats were randomly assigned to either a T9 spinal cord injury or sham laminectomy. Locomotion recovery was assessed using the Basso, Beattie, and Bresnahan open field rating scale and the CatWalk XT■gait analysis. To determine the potential bias from weight changes, corrected hindlimb(H) values(divided by the unaffected forelimb(F) values) were calculated. Six weeks after injury, cyst formation, astrogliosis, and the deposition of chondroitin sulfate glycosaminoglycans were assessed by immunohistochemistry staining. Compared with the baseline, a significant spontaneous recovery could be observed in the CatWalk XT■parameters max intensity, mean intensity, max intensity at%, and max contact mean intensity from 4 weeks after injury onwards. Of note, corrected values(H/F) of CatWalk XT■parameters showed a significantly less vulnerability to the weight changes than absolute values, specifically in static parameters. The corrected CatWalk XT■parameters were positively correlated with the Basso, Beattie, and Bresnahan rating scale scores, cyst formation, the immunointensity of astrogliosis and chondroitin sulfate glycosaminoglycan deposition. The CatWalk XT■gait analysis and especially its static parameters, therefore, seem to be highly useful in assessing spontaneous recovery of hindlimb function after severe thoracic spinal cord injury. Because many CatWalk XT■parameters of the hindlimbs seem to be affected by body weight changes, using their corrected values might be a valuable option to improve this dependency.
文摘Nowadays, assessing speaking has received a great degree of attention not only because it is difficult to develop and administer, but also it plays a significant role in eliciting the purpose of foreign language learning. Studies on assessing speaking explored different proposals influencing the test of speaking, such as the nature of speaking, basic types of speaking, mode of test and rating scales. This study compares different proposals for assessing speaking in foreign language learning. Deep analyses according to Beijing Oral English Certificate(BOEC) testing will be presented with the improvement suggestions.
基金funding from NIMH, AHRQ, Marriott Foundation, Mayo Foundation
文摘AIM: To investigate child and adolescent psychiatrists'(CAPs) attention deficit hyperactivity disorder(ADHD) and oppositional defiant disorder(ODD) diagnoses and treatments in real-world clinical practice. METHODS: The medical records of 69 ADHD children(mean age = 9.5 years), newly referred to the ADHD clinic, were reviewed for their scores of parent- and teacher-reported Vanderbilt ADHD Diagnostic Rating Scales(VADRSs), CAPs' diagnoses of ADHD and ODD, and CAPs' treatment recommendations. Among 63 ADHD subjects who completed both parent and teacher VADRSs, we examined the agreement of the parent and teacher VADRSs. We also examined the concurrent validity of CAPs' ODD diagnoses against the results from the VADRSs. In addition, we compared CAPs' treatment recommendations against established ADHD and ODD guidelines.RESULTS: Among 63 ADHD subjects, the majority of the subjects(92%) met full ADHD diagnostic criteria at least in one setting(parent or teacher) on the VADRSs. Nearly half of the patients met full ADHD diagnostic criteria in two settings(parent and teacher). Relatively low agreement between the parent and teacher VADRSs were found(95%CI:-0.33 to 0.14). For 29 children who scored positive for ODD on the rating scales, CAPs confirmed the ODD diagnosis in only 12 of these casepositives, which is considered as a fair agreement between CAPs and VADRSs(95%CI: 0.10-0.53). For 27 children with no ODD diagnosis made by either CAP or VADRS, more than half of them were recommended for medication only. In contrast, where CAPs made the diagnosis of ODD, or where the parent or teacher VADRS was positive for ODD, almost all of the patients received recommendations for medication and behavior therapy.CONCLUSION: CAPs' ADHD diagnoses have strong concurrent validity against valid rating scales, but ADHD's most common comorbid condition- ODD- may be underrecognized.
文摘Few experimental studies have evaluated the efficacy of continuing educational programs aimed at the improvement of nurses’ pain-management skills. This study assessed whether a standardized educational program aimed at nurses could increase the use of the Numeric Rating Scale-11 in both documenting and reducing postoperative pain-intensity levels in hospitalized surgical patients. The study had a quasi-experimental pre- and post-intervention design. Data were collected from records of surgical patients prior to and after the standardized educational program was completed. There were no significant differences between pre- and post-intervention groups in terms of either pain-documentation frequency or pain-intensity level. The study showed no increase in the frequency of postoperative pain documentation and no reduction of surgical patients’ postoperative pain-intensity level. This finding indicates that the standardized educational program on postoperative pain management was insufficient to bring about changes in clinical practice.
文摘Ataxias are rare diseases and the etiologic heterogeneity make individual entities even rarer.There are still substantial numbers of patients who are still poorly understood.Available assessment techniques still point to large numbers of patients needed for clinical trials and the need for cooperative efforts,better assessment tools and novel trial designs.Better understanding of neural circuitry abnormalities may lead to more effective symptomatic therapy.Opportunities exist for targeting at risk individuals for effective therapies but how this can be done is not clear.Preventive strategies may become feasible in many ataxias.
文摘Objective:To study the therapeutic effect of Shangjinbitong Powder(伤筋痹痛散)on improving acupoint plaster,in order to provide some scientific basis for clinical use.Methods:This project was carried out from June 2020 to January 2021,30 patients with low back pain in the outpatient department of orthopedics and traumatology in our hospital and 30 patients with low back pain in the rehabilitation department of Fuping County Hospital of Traditional Chinese Medicine were selected as the research objects.Patients with low back pain in our hospital were treated with Shangjinbitong Powder as the control group,and patients in rehabilitation department of Fuping County Hospital of Traditional Chinese Medicine as the research group were treated with acupoint plaster improved by Shangjinbitong Powder.After one week of treatment,the effective rates of the two groups were compared.Results:The effective rate of treatment in the study group was significantly higher than that in the control group(P<0.05).There were no adverse reactions such as infection,scald and bleeding in the study group and the control group during the treatment.Conclusion:Shangjinbitong Powder improved acupoint plaster has good curative effect,convenient use,certain safety and clinical popularization value.
基金This study was supported by grants from the National Natural Science Foundation of China grant,The Innovation Team Fund of the First Affiliated Hospital of Zhengzhou University and the National Natural Science Foundation of China
文摘Background: Spinocerebellar ataxia type 3 (SCA3) is the most common subtype of SCA worldwide, and runs a slowly progressive and unremitting disease course. There is currently no curable treatment available. Growing evidence has suggested that nerve growth factor (NGF) may have therapeutic effects in neurodegenerative diseases, and possibly also in SCA3. The objective of this study was to test the efficacy of NGF in SCA3 patients. Methods: We performed an open-label prospective study in genetically confirmed adult (〉18 years old) SCA3 patients. NGF was administered by intramuscular injection (18 μg once daily) fbr 28 days consecutively. All the patients were evaluated at baseline and 2 and 4 weeks after treatment using the Chinese version of the scale for assessment and rating of ataxia (SARA). Results: Twenty-one SCA3 patients ( 10 men and 11 women, mean age 39.14 ± 7.81 years, mean disease duration 4.14 ± 1.90 years, mean CAG repeats number 77,57 ± 2.27) were enrolled. After 28 days of NGF treatment, the mean total SARA score decreased significantly from a baseline of 8.48± 2.40 to 6.30 ± 1.87 (P 〈 0.001 ). Subsections SARA scores also showed significant improvements in stance (P = 0.003), speech (P = 0.023), finger chase (P = 0.015), fast alternating hand movements (P = 0.009), and heel-shin slide (P = 0.001). Conclusions: Our preliminary data suggest that NGF may be effective in treating patients with SCA3.
基金National Natural Science Foundation of China:Research on the Neuroprotective Mechanism of the Gandouling based on the Regulation of Long Non-coding Ribonucleic Acid Maternally Expressed Gene 3 and Targets Gene Forkhead Box O1 through the Way of Autophagy in Wilson Disease(No.82104783)National Natural Science Foundation of China:Study on Mechanism of Xin'an Medical Gubenpeiyuan Prescription Treating Wilson's Disease based on Enterohepatic Axis and Enterobrain Axis Pathway(No.U22A20366)+2 种基金National Administration of Traditional Chinese Medicine:2019 Project of Building Evidence based Practice Capacity for Traditional Chinese Medicine(No.2019XZZX-NB001)National Natural Science Foundation of Anhui Province:Mechanism Study on the Regulation of Peroxisome Proliferator-activated Receptor-gamma Coactivator-1alpha/Sirtuin-3/Mitochondrial Axis to Maintain Hippocampal Synaptic Homeostasis to Improve Cognitive Impairment in Wilson's Disease by Qingre Lidan JieDu Prescription(No.2008085MH264)National Natural Science Foundation of Anhui Province:Mitofusin 2 Mediates the Role of Endoplasmic Reticulum-MAMs-mitochondria Regulation of Synaptic Homeostasis in Wilson's Disease Neurons and the Mechanism of Qingre Lidan Jiedu Prescription Intervention(No.2208085MH271)。
文摘OBJECTIVE:To investigate the value of relative exchangeable copper(REC)in diagnosing Wilson's disease(WD)and to determine its significance in the differentiation of Traditional Chinese Medicine(TCM)syndrome.METHODS:A total of 78 patients with WD were recruited on the same day of the medical visit,and among them,32 were suffering from non-WD(N-WD)and 37 were heterozygous ATP7B carriers(HC)enrolled as controls.Molecular genetic testing was performed for diagnosing WD and HC.Additionally,REC levels in different TCM syndromes were investigated.The correlation between REC and serum ceruloplasmin(Cp),serum copper oxidase(CO),and 24-h urinary copper was analyzed using the Global Assessment Scale and Unified Wilson's Disease Rating Scale and the significance of REC in WD diagnosis was investigated.Resting-state functional magnetic resonance imaging was used to assess the clinical symptoms of WD and analyze its severity in different TCM syndromes.RESULTS:REC determination helped in significantly distinguishing patients with N-WD or HC from those with WD with a cut-off of 21.15%.Furthermore,the comparative analysis of REC ratios among different TCM syndromes showed markedly high REC levels in the dampness-heat internal accumulation syndrome group.Additionally,the seed-based functional connectivity value from the putamen to the cerebellum was significantly correlated with TCM syndromes.CONCLUSION:REC level is a high-quality biomarker for diagnosing WD that effectively reflects disease severity and plays an essential role in deciding on treatment strategies and prognosis.Furthermore,REC levels are closely related to TCM syndromes in WD;thus,it is a potential objective quantitative indicator for distinguishing TCM syndromes in WD.
文摘Objective:Vulvar lichen planus(VLP),especially erosive VLP(EVLP),seriously affects women’s physiological function and quality of life.A retrospective analysis of erosive vulvar lichen planus(EVLP)was performed to raise awareness on this chronic,deforming,and painful vulvar disease.Methods:This observational retrospective study involved 40 patients with EVLP among 1,368 patients with vulvar disease from 2017 to 2020.The patients’vulvar clinical features and extravulvar mucosal involvement were investigated.The patients’pain and quality of life were evaluated with the numeric rating scale and the Dermatology Life Quality Index(DLQI),respectively.Clinical characteristics were reported using frequencies and proportions for categorical variables.Continuous variables are expressed as mean±standard deviation.The association between NRS score for pain and DLQI score was analyzed by Pearson’s correlation test.Results:EVLP accounted for 2.9%(40/1,368)of all cases of vulvar disease,and the median age at onset was 42 years.Erosion mostly occurred on the labia minora and introitus.Half(20/40)of the patients had extravulvar mucosal involvement.Seventeen(42.5%)patients had oral lichen planus,and seven(17.5%)patients were diagnosed with vulvovaginal-gingival syndrome.The numeric rating scale score showed that 47.5%(19/40)of patients experienced mild pain,whereas 12.5%(5/40)described the pain as severe.Thirty-one patients(77.5%,31/40)had dyspareunia,and nine patients had aggravated pain after sexual intercourse.The mean DLQI score was 9.1±6.0,with 37.5%(15/40)of patients indicating that EVLP had severely affected their lives.The numeric rating scale score for pain was significantly correlated with the DLQI score(P=0.007).Conclusion:EVLP can affect the quality of women’s lives and cause severe deformations and painful architectural changes.The description of the characteristics of EVLP in this report will benefit the diagnosis and early treatment of this disease.